Immunodeficiency Evaluation for Chronic Infections in Infants and Children Testing
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Recurrent respiratory infections with or without chronic diarrhea Severe viral infections; Candida or other fungal infections; recurrent sinopulmonary infections Abscesses, pneumonia, delayed separation of umbilical cord; recurrent respiratory infections with or without diarrhea CONSIDER Immunoglobulins (IgA, IgG, IgM), Quantitative Lymphocyte Subset Panel 6 - Total Lymphocyte Enumeration with CD45RA and CD45RO or Lymphocyte Subset Panel 7 Congenital Immunodeficiencies Response to polyvalent pneumococcal vaccine if >2 years Response to DT vaccine Sweat Chloride testing (at accredited cystic fibrosis center) low IgG, IgM, IgA OR poor antibody response to vaccination ORDER B-Cell Immunodeficiency Profile no B-cells present Bruton X-linked agammaglobulinemia high IgM low IgG, IgA B-cells present Common variable immune deficiency (CVID) or other immune deficiency CD 40 Ligand testing Possible Hyper IgM Syndrome Immunology consult May need immunoglobulin replacement positive sweat chloride Cystic fibrosis low IgA ORDER Immunoglobulin G Subclasses (1, 2, 3, 4) IgA absent Low IgG 2,4 Poor pneumococcal vaccine response Immunology consult May need immunoglobulin replacement with low IgA containing preparation IgA absent Normal IgG subclasses Warn of possible reaction to IgA- containing blood products Symptomatic treatment Test of choice in children 18 months is HIV PCR (antibodies do not work in infants) ORDER Lymphocyte Subset Panel 6 - Total Lymphocyte Enumeration with CD45RA and CD45RO or Lymphocyte Subset Panel 7 Congenital Immunodeficiencies AND Lymphocyte Antigen and Mitogen Proliferation Panel low T-cell low LAM abnormal facies hypocalcemia Possible severe combined immunodeficiency ORDER Adenosine Deaminase, RBC Purine Nucleoside Phosphorylase Toll-Like Receptor Function Also consider IL-2R gamma chain defect or other related diseases low T-cell low LAM DiGeorge syndrome HIV positive See HIV algorithms Decreased response to Candida ORDER Toll-Like Receptor Function Assay Possible chronic mucocutaneous candidiasis ORDER Immunoglobulins (IgA, IgG, IgM), Quantitative Complement Activity Enzyme Immunoassay, Total and AH50 CBC with Platelet Count and Automated Differential Neutrophil Oxidative Burst Assay (DHR) Leukocyte Adhesion Deficiency Panel Myeloperoxidase Stain Lymphocyte Subset Panel 7 Congenital Immunodeficiencies low IgG or IgM Hypogammaglobulinemia abnormal complement activity Possible complement deficiency Genetic testing increased IgE Possible Hyper IgE syndrome (Job syndrome) Candida specific IgE neutrophil chemotaxis low neutrophil count Kostmann agranulocytosis Hematology consult May need granulocyte colony stimulating factor Neutrophil antibody positive Autoimmune neutropenia 2006 ARUP Laboratories. All Rights Reserved. Revised 01/19/2012 www.arupconsult.com ORDER Chromosome FISH, Metaphase (specify 22q11 deletion) positive for deletion ORDER B-Cell Immunodeficiency Profile normal IgM cells, low IgG and IgA all normal Innate immune deficiency absence of myeloperoxidase Myeloperoxidase deficiency ORDER Individual complement testing based on results of CH50, AH50 If all normal, contact immunology director for further evaluation T-cell disorder evaluation Immunoglobulin disorder evaluation IgA low Normal IgG subclasses Consider celiac testing abnormal DHR Chronic granulomatous disease decreased CD11b/CD 18 Leukocyte adhesion deficiency, type 1 decreased CD15 Leukocyte adhesion deficiency, type 2 ORDER Genetic testing ORDER IRAK-4 and toll receptor abnormal